Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency

  title={Inhibition of Mitochondrial Aconitase by Succination in Fumarate Hydratase Deficiency},
  author={Nicola Ternette and Ming Yang and Mahima Laroyia and Mitsuhiro Kitagawa and Linda O’Flaherty and Kathryn Wolhulter and Kaori Igarashi and Kaori Saito and Keiko Kato and Roman Fischer and Alexandre Berquand and Benedikt M. Kessler and Terry Lappin and Norma Frizzell and Tomoyoshi Soga and Julie Adam and Patrick J. Pollard},
  booktitle={Cell reports},
The gene encoding the Krebs cycle enzyme fumarate hydratase (FH) is mutated in hereditary leiomyomatosis and renal cell cancer (HLRCC). Loss of FH activity causes accumulation of intracellular fumarate, which can directly modify cysteine residues to form 2-succinocysteine through succination. We undertook a proteomic-based screen in cells and renal cysts from Fh1 (murine FH)-deficient mice and identified 94 protein succination targets. Notably, we identified the succination of three cysteine… CONTINUE READING
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the result of the oxidation of the catalytic site cysteine to cysteine-sulfinic acid

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